0521779407-16 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:18
Other Clotting Factor Deficiencies 1117■HCV RNA PCR at baseline, evaluate for biopsy, hepatitis Rx
■CD4, HIV RNA PCR q 3–6 months, evaluate for antiretroviral Rx
complications and prognosis
Complications
■at birth, CNS bleeding, intracranial bleeding, cephalhematoma,
especially with forceps, vacuum extraction
■at diagnosis, bleeding with procedures, surgery, when no family his-
tory taken or no factor replacement given
■bleeding associated with drugs – e.g., NSAIDs, aspirin, or analgesics,
platelet inhibitory drugs, Coumadin, antibiotics
■bleeding associated with other disorders that cause platelet dysfunc-
tion – e.g., uremia, ITP, liver disease, portal hypertension
■bacterial infection of body cavity hematomas
■compartment syndrome due to severe, large hemorrhage – e.g., of
the extremities
■entrapment neuropathies associated with large hematomas (reto-
peritoneal)
■thrombosis, hepatitis complications from blood products, transfu-
sions orthopedic, soft tissue, neurologic complications of hemorr-
hages: headaches, mental status change, or peripheral neuropathies
associated with hematomas
■traumatic bleeds which block airway, causing mental status change,
CNS bleed, cord compression
■anemia, iron deficiency, transfusion requirement, when preopera-
tive treatment is inadequate by product, dose, or duration
■AIDS among those with chronic HIV infection
■end-stage liver disease, drug interactions, or hepatocellular carci-
noma among those with chronic HCV infectionPrognosis
■homozygotes may die at birth or shortly thereafter
■heterozygotes may be symptomatic with trauma, procedures; most
live normal life with adequate treatment, followup
■women with menorrhagia may develop iron deficiency anemia,
undergo unnecessary gynecologic procedures, and more bleeding
■recurrent bleeding into joints in those with severe factor deficiency
may lead to chronic arthritis and disability
■shortened lifespan, morbidity in areas without expertise in coagula-
tion